Right Ventricular Function Returns in Children Undergoing Double Lung Transplant for PAH

The authors said their findings present a better option for pediatric patients with severe disease.

A recent study showed that children undergoing bilateral lung transplantation for severe pulmonary arterial hypertension (PAH) had a full recovery of their right ventricular (RV) systolic function at the 6-week mark.

Recovery happened within 2 months, wrote the authors, and the results were independent of the patients’ age, weight, and hemodynamic impairment.

The authors used advanced echocardiography and cardiac MRI, followed by conventional and RV strain analysis, pre- and posttransplant, in an effort to clarify some beliefs about this patient population.

The timing of transplants in these children remains challenging, the authors said. Many are referred late or not at all, due to the belief that the transplant operation would not be successful long term in patients with impaired RV function or that their young age and low body weight would prove too challenging to overcome.

This prospective observational study involved 15 patients, aged 1.9 to 17.6 years, undergoing a double lung transplant at Hannover Medical School in Germany between December 2013 and January 2021. Only patients with primary PAH were included, as defined by the World Symposium on Pulmonary Hypertension (PH):

  • Mean pulmonary arterial pressures: > 20 mm Hg
  • Pulmonary arterial wedge pressure : ≤ 15 mm Hg
  • Pulmonary vascular resistance index: ≥3 WU·m2 when > 3 months, at sea level.

The European Pediatric Pulmonary Vascular Disease Network PH risk score was used to judge the patients’ condition before surgery.

The prospective observational imaging study showed the rapid and full recovery of RV systolic function within 6 weeks, irrespective of the severity of RV dysfunction, age, weight, or need for veno-arterial extracorporeal membrane oxygenation (VA-ECMO) before transplant (the majority of the patients were transplanted while on VA-ECMO).

There was a complete normalization of RV ejection fraction, improving from 30% to 63%, as determined by cardiac MRI. In addition, in contrast to the persistently increased RV mass, RV volumes rapidly normalized.

“Even in end-stage pediatric PAH with poor RV function and low cardiac output, transplant should be preferred over heart-lung transplantation,” wrote the authors.

Reference

Hansmann G, Diekmann F, Chouvarine P, et al. Full recovery of right ventricular systolic function in children undergoing bilateral lung transplantation for severe PAH. J Heart Lung Transplant. 2022;41(2):187-198. doi:10.1016/j.healun.2021.10.014